Age of Onset and Disease Course in Biopsy-Proven Minimal Change Disease: An Analysis From the Cure Glomerulonephropathy Network.
American journal of kidney diseases : the official journal of the National Kidney Foundation
washington; spokane; pmrc
RATIONALE & OBJECTIVE: Adolescent- and adult-onset minimal change disease (MCD) may have a clinical course distinct from childhood-onset disease. We characterized the course of children and adults with MCD in the Cure Glomerulonephropathy Network (CureGN) and assessed predictors of rituximab response.
STUDY DESIGN: Prospective, multicenter, observational study.
STUDY PARTICIPANTS: CureGN participants with proven MCD on biopsy.
EXPOSURE: Age at disease onset, initiation of renin-angiotensin-aldosterone system (RAAS) blockade, and immunosuppression including rituximab during the study period.
OUTCOME: Relapse and remission, change in estimated glomerular filtration rate (eGFR), and kidney failure.
ANALYTICAL APPROACH: Remission and relapse probabilities were estimated using Kaplan-Meier curves and gap time recurrent event models. Linear regression models were used for the outcome of change in eGFR. Cox proportional hazards models were used to estimate the association between rituximab administration and remission.
RESULTS: The study included 304 childhood- (≤12 years old), 49 adolescent- (13-17 years old), and 201 adult- (≥18 years) onset participants with 2.7-3.2 years of follow-up after enrollment. Children had a longer time to biopsy (238 vs 23 and 36 days in adolescent- and adult-onset participants, respectively; P
LIMITATIONS: CureGN was limited to biopsy-proven disease. Comparisons of childhood to nonchildhood cases of MCD may be subject to selection bias, given that childhood cases who undergo a biopsy may be limited to patients who are least responsive to initial therapy.
CONCLUSIONS: Among patients with MCD who underwent kidney biopsy, there were differences in the course (relapse and remission) of childhood-onset compared with adolescent- and adult-onset disease, as well as rituximab response.
PLAIN-LANGUAGE SUMMARY: Minimal change disease is a biopsy diagnosis for nephrotic syndrome. It is diagnosed in childhood, adolescence, or adulthood. Patients and clinicians often have questions about what to expect in the disease course and how to plan therapies. We analyzed a group of patients followed longitudinally as part of the Cure Glomerulonephropathy Network (CureGN) and describe the differences in disease (relapse and remission) based on the age of onset. We also analyzed rituximab response. We found that those with childhood-onset disease had a higher rate of relapse but also have a higher probability of reaching remission when compared with adolescent- or adult-onset disease. Children and all steroid-responsive patients are more likely to achieve remission after rituximab.
Kidney & Diabetes
Chen, Dhruti P; Helmuth, Margaret E; Smith, Abigail R; Canetta, Pietro A; Ayoub, Isabelle; Mucha, Krzysztof; Kallash, Mahmoud; Kopp, Jeffrey B; Gbadegesin, Rasheed; Gillespie, Brenda W; Greenbaum, Larry A; Parekh, Rulan S; Hunley, Tracy E; Sperati, C John; Selewski, David T; Kidd, Jason; Chishti, Aftab; Reidy, Kimberly; Mottl, Amy K; Gipson, Debbie S; Srivastava, Tarak; Twombley, Katherine E; Consortium, CureGN; and Tuttle, Katherine, "Age of Onset and Disease Course in Biopsy-Proven Minimal Change Disease: An Analysis From the Cure Glomerulonephropathy Network." (2023). Articles, Abstracts, and Reports. 7410.